Background/Aims  According to available data children with SARS-CoV-2 has asymptomatic or a milder clinical course, but there are known cases of multisystem inflammatory syndrome. Methods  we observed a case of fulminant purpura in a boy with SARS-CoV-2. A three years old boy had febrile fever, vomiting, abdominal pain for no apparent reason. On the 2nd day this boy had joint pain, petechial rashes, fever and abdominal pain persisted. The patient was hospitalized in the pediatric Department, where he was diagnosed with hemorrhagic vasculitis, there was prescribed therapy with glucocorticosteroids (1 mg/kg per day), dipyridamole (2.5 mg/kg per day), oral cephalosporin. Against the background of ongoing therapy, the child's condition became worth. On the 5th day of the disease appeared necrosis in the area of the ankle joints, heels, and toes, patient was transferred to the pediatric Department of Children's Clinical Regional Hospital. Results  at the time of hospitalization, the child's condition was severe: the boy had persistent high fever, petechial rash on the torso and legs, necrosis in the area of the left ankle, outer surface of the right ankle and in the area of both heels, edema of the feet and hands. The boy was in a forced position, couldn’t not walk and had intense pain in the shoulders, elbows, wrists, hips, knees and ankles. Blood tests revealed anemia (HGB 73 g/l), thrombocytopenia (PLT 120 x 109/L), leukocytosis (WBC 23 x 109/L), ESR 60 mm/h; in the clinical analysis of urine - proteinuria. In the blood test was detected significantly increased levels of CRP (129 mg/L), ferritin (637 ng/mL), triglycerides (1.39 mm/L), hypoproteinemia (48 g/L), hypoalbuminemia (31 g/L); the level of ALT, AST, LDH was normal. The level of D-dimer was 4792ng/mL, fibrinogen - 8.8 g/L, antithrombin III - 156%, protein C - 64%, coagulation Factor IX activity - 150 %, coagulation Factor VIII activity 289%. An increase of antinuclear antibodies, cardiolipins, phospholipids and ANCA were excluded. A nasopharyngeal swab was negative for SARS-CoV-2 (was made on the 5th day of the disease). On 14th illness day we detected IgM antibodies directed toward SARS-CoV-2 in this patient (IgG antibodies directed toward SARS-CoV-2 were absent). An echocardiogram showed no coronary artery abnormalities, echocardiogram was normal, a chest CT and MRI of the brain also were normal. The diagnosis was fulminant purpura in a patient with SARS-CoV-2. This boy received heparin, IVIG 2 g/kg, dexamethasone 10 mg per meter of body surface a day, antibiotic therapy was continued. As a result fever, petechial rash and necrosis regressed. After 4 weeks we detected IgG antibodies directed toward SARS-CoV. Conclusion  the current case demonstrates the possibility of developing a multi-system inflammatory syndrome like fulminant purpura in patients who had SARS-CoV-2. Disclosure  N. Tsurikova: None. S. Rodionocskaya: None. E. Ligostaeva: None. V. Avdeenko: None. N. Kobzeva: None. I. Tsiganok: None.

Background/Aims  During the COVID-19 pandemic, analysis of the incidence of COVID-19 among patients suffering from rheumatic diseases and receiving therapy with biological agents remains relevant. Methods  This single-center observational study included 118 children suffering from various rheumatic diseases and receiving therapy with anti-rheumatic drugs and biological agents. In this research, we analyzed the incidence of CIVID-19 and the frequency of documented contact with SARS-CoV-2 in the period from 01.03.2020 to 11.10.2020 (32 weeks). The results were analyzed using descriptive statistics. Results  Among 118 children, there were 28 (24%) boys and 90 (76%) girls, average age 10.3±4.2. 104 (88.2%) patients had different types of juvenile idiopathic arthritis (JIA), 2 (1.6%) children had systemic lupus erythematosus (SLE), 2 (1.6%) patients had juvenile dermatomyositis (JDM), 1 (1%) child had ANCA-associated vasculitis, 6 (5%) patients had familial Mediterranean fever (FMF), 2 (1.6%) children had deficiency of adenosine deaminase 2 (DADA2), 1 (1%) child had TNF receptor-associated periodic syndrome (TRAPS). In this group of patients 94 (79%) patients were treated with methotrexate, 1 (1%) - azathioprine, 3 (2%) patients received hydroxychloroquine, 6(5%) - mycophenolate mofetil, 4 (3%) - sulfasalazine, 14(11%) children received prednisone, 6(5%) - cyclosporine A. All children included in this study received biological agents for more than 1 year, the distribution of biological agents among patients was as follows: 41(34%) - etanercept, 33(28%) - adalimumab, 24 (20%) - tocilizumab, 7 (6%) - canakinumab, 3 (2%) - abatacept, 4 (3%) - golimumab, 6 (5%) - rituximab. Out of 118 children, 4 (3%) patients had flu-like symptoms and positive results of PCR tests for COVID-19 (1 patient was treated with etanercept, 1 - adalimumab, 1 - tocilizumab, 1 - rituximab), none of the patients had signs of SARS-CoV-2 pneumonia. 10 (8%) patients had documented contact with COVID-19: among this patients 2 children had flu-like symptoms, positive results of PCR tests and absence of COVID-19 pneumonia (one of this patient was treated with adalimumab, another one - with rituximab), one more patient was treated with tocilizumab and had positive PCR test without any symptoms of COVID-19; other 7 children had negative PCR tests and didn’t have any signs of COVID-19. Conclusion  Among our patients with various rheumatic diseases treated with biological agents there were no registered severe cases of COVID-19. Over the past period (32 weeks of follow-up) 3% of children with COVID-19 were identified and 8% patients had documented contact with COVID-19, but we suppose it is too early to make conclusions about the degree and severity of COVID-19 among children suffering from rheumatic diseases and receiving various biological agents. Further follow-up is needed to better understand the risk and impact of COVID-19 among children with rheumatic diseases and receiving therapy with biological agents. Disclosure  N. Tsurikova: None. E. Ligostaeva: None. V. Avdeenko: None. N. Kobzeva: None. I. Tsiganok: None. K. Skorobogatova: None. A. Motkina: None.

Областная детская клиническая больница, Ростов-на-Дону, Российская Федерация Обоснование. Колхицинрезистентные случаи семейной средиземноморской лихорадки (ССЛ) сопряжены с высоким риском развития амилоидоза почек и тяжелым течением приступных периодов болезни, характеризующихся фебрильной лихорадкой, артритами, плевритами, перикардитами. Контроль болезни у колхицинрезистентных больных труднодостижим и требует использования генно-инженерных биологических препаратов. Описание клинического случая. Представлено наблюдение семейного случая тяжелого течения ССЛ. Описано успешное применение моноклонального антитела к интерлейкину 1 канакинумаба у сестер с колхицинрезистентной формой заболевания. Уже через 4 нед лечения были полностью купированы лихорадка, суставной и абдоминальный синдромы, отмечена нормализация лабораторных признаков воспаления. Через 32 нед терапии лабораторные показатели активности болезни (скорость оседания эритроцитов и концентрация С-реактивного белка) сохранялись в пределах референсных значений, приступов болезни не было. За период наблюдения (32 нед) на фоне терапии канакинумабом развития нежелательных реакций не отмечено. Заключение. Показана высокая эффективность канакинумаба у пациентов с тяжелым течением ССЛ, резистентной к терапии колхицином.

No abstract